Diagnostic criteria for cryopyrin-associated periodic syndrome (CAPS)
Cryopyrin-associated periodic syndrome (CAPS) is a rare, heterogeneous disease entity
associated with NLRP3 gene mutations and increased interleukin-1 (IL-1) secretion. Early …
associated with NLRP3 gene mutations and increased interleukin-1 (IL-1) secretion. Early …
Sex differences in pediatric rheumatology
M Cattalini, M Soliani, MC Caparello… - Clinical reviews in allergy & …, 2019 - Springer
Autoimmune diseases affect up to 10% of the world's population and, as a whole, they are
far more common in females, although differences exist according to the single disease and …
far more common in females, although differences exist according to the single disease and …
Behçet's disease in children, an overview
I Koné-Paut - Pediatric Rheumatology, 2016 - Springer
BD is a systemic inflammatory disease with a variable vasculitis. Paediatric onset is very rare
and carries a strong genetic component. Oral ulcers and fever of unknown origin are …
and carries a strong genetic component. Oral ulcers and fever of unknown origin are …
A20 haploinsufficiency (HA20): clinical phenotypes and disease course of patients with a newly recognised NF-kB-mediated autoinflammatory disease
FA Aeschlimann, ED Batu, SW Canna, E Go… - Annals of the …, 2018 - ard.bmj.com
Objectives The association between mutations in TNFAIP3, encoding the NF-kB regulatory
protein A20, and a new autoinflammatory disease has recently been recognised. This study …
protein A20, and a new autoinflammatory disease has recently been recognised. This study …
Changes in the proportion of clinical clusters contribute to the phenotypic evolution of Behçet's disease in Japan
Abstract Background We hypothesized that Behçet's disease (BD) consists of several clinical
subtypes with different severity, resulting in heterogeneity of the disease. Here, we …
subtypes with different severity, resulting in heterogeneity of the disease. Here, we …
French recommendations for the management of Behçet's disease
I Kone-Paut, S Barete, B Bodaghi, K Deiva… - Orphanet journal of rare …, 2021 - Springer
Behçet's disease (BD) is a systemic variable vessel vasculitis that involves the skin, mucosa,
joints, eyes, arteries, veins, nervous system and gastrointestinal system, presenting with …
joints, eyes, arteries, veins, nervous system and gastrointestinal system, presenting with …
Pediatric Behçet's disease
Behçet's Disease (BD) is a systemic vasculitis firstly described as a disorder causing
aphthous lesion in oral and genital mucosae and uveitis. The disease has an extremely …
aphthous lesion in oral and genital mucosae and uveitis. The disease has an extremely …
Intestinal Behçet and Crohn's disease: two sides of the same coin
S Valenti, R Gallizzi, D De Vivo, C Romano - Pediatric Rheumatology, 2017 - Springer
Behçet's disease (BD) and Crohn's disease (CD) are chronic immune-mediated,
inflammatory disorders affecting many different systems (joints, skin, eyes, gastrointestinal …
inflammatory disorders affecting many different systems (joints, skin, eyes, gastrointestinal …
Childhood vasculitis
A Schnabel, CM Hedrich - Frontiers in pediatrics, 2019 - frontiersin.org
The term vasculitis covers heterogeneous disorders that share the presence of inflammation
of blood vessel walls. Immune cell infiltrates can vary significantly and involve granulocytes …
of blood vessel walls. Immune cell infiltrates can vary significantly and involve granulocytes …
Clinical characteristics and genetic analyses of 187 patients with undefined autoinflammatory diseases
NM Ter Haar, C Eijkelboom, L Cantarini… - Annals of the …, 2019 - ard.bmj.com
Objectives To describe the clinical characteristics, treatment response and genetic findings
in a large cohort of patients with undefined systemic autoinflammatory diseases (SAIDs) …
in a large cohort of patients with undefined systemic autoinflammatory diseases (SAIDs) …